Neurologic complications are a major cause of morbidity and mortality in sickle
cell disease (SCD). In children with sickle cell anemia, routine use of transcranial
Doppler screening, coupled with regular blood transfusion therapy, has decreased
the prevalence of overt stroke from ∼11% to 1%. (Blood. 2015;125(22):3401-3410)
BLOOD, 28 MAY 2015 x VOLUME 125, NUMBER 22
Sickle cell disease (SCD) affects 12 000–15 000 individuals in the UK. Whilst homozygous SCD (sickle cell anaemia — HbSS) is the most common and severe genotype, and is where most of the evidence exists, this guidance should be used for all genotypes of SCD
British Journal of Haematology, 2015, 169, 492–505 doi: 10.1111/bjh.13348
הנחיות האיגוד הישראלי להמטולוגיה ואונקולוגיה ילדים 2014
Renal disease is one of the most frequent and severe complications experienced by patients with sickle cell disease; its prevalence is likely to increase as the patient
BLOOD, 12 JUNE 2014 x VOLUME 123, NUMBER 24
- SCD affects the entire body, beginning in very early infancy, and a multidisciplinary team is needed to care for children with SCD.
- Some forms of SCD-related morbidity, such as overt stroke, are decreasing owing to prognostic and therapeutic advancements.
- Primary prevention of organ injury is an important focus of current research.
- Almost all children born with SCD in developed nations now survive to adulthood, but the transition to adult medical care is a high-risk period for death.
Pediatr Clin N Am 60 (2013) 1363–1381 http://dx.doi.org/10.1016/j.pcl.2013.09.006
- The thalassemia syndromes are a heterogeneous group of disorders characterized by variable degrees of hemolysis, chronic anemia, and ineffective erythropoiesis.
- Because more patients are living longer, disease- and treatment-related complications are becoming more common.
- Optimal and safe transfusion support, iron chelation, noninvasive iron assessments, and stem cell therapies provide new tools for effective management of thalassemia.
Pediatr Clin N Am 60 (2013) 1383–1391 http://dx.doi.org/10.1016/j.pcl.2013.08.008
Patients with b-thalassemia major (TM) and other refractory anemias requiring regular blood transfusions accumulate iron that damages the liver, endocrine system, and most importantly the heart. The prognosis in TM has improved remarkably over the past 10 years.
BLOOD, 1 NOVEMBER 2012 | VOLUME 120, NUMBER 18
The purpose of this article is to set forth our approach to diagnosing and managing the
thalassemias, including b-thalassemia intermedia and b-thalassemia major.
BLOOD, 29 SEPTEMBER 2011 | VOLUME 118, NUMBER 13
Acute chest syndrome describes new respiratory symptoms and findings, often severe and progressive, in a child with sickle cell disease and a new pulmonary infiltrate.
BLOOD, 19 MAY 2011 | VOLUME 117, NUMBER 20 5297
British Journal of Haematology, 149, 35–49, doi:10.1111/j.1365-2141.2009.08054.x